Search results for " Progressive"

Dentatorubral-pallidoluysian atrophy: haplotype of Asian origin in 2 Italian families.

2011

Formation of basement membrane in extracapillary proliferates in rapidly progressive glomerulonephritis

1977

In the extracapillary proliferations (crescents) of the glomeruli in glomerulonephritis, basement membranes appear and in addition "secretory bodies" are formed in the cisternae of the rough endoplasmatic reticulum. The findings permit the conclusion that proliferated visceral epithelial cells are involved in the crescent formation to a greater extent than previously assumed.

Progressive multifocal encephalopathy in a patient with non-Hodgkin follicular lymphoma

2020

Progressive multifocal leukoencephalopathy (PML) is a rare and often fatal demyelinating disease of the central nervous system caused by John Cunningham virus (JCV). We present a case report of patient with non-Hodgkin follicular lymphoma, who developed PML after hematopoietic stem cell transplantation and rituximab-bendamustine therapy. JCV DNA was proven both in peripheral blood and cerebrospinal fluid. Patient with 4 years history of follicular lymphoma presented with progressing weakness in the right arm and leg and postural instability. Magnetic resonance imaging scans showed bilateral hyperintense lesions in the cerebellum and centrum semiovale consistent with findings in PML. JCV DNA…

Multiple sclerosis patient-derived CSF induces transcriptional changes in proliferating oligodendrocyte progenitors.

2014

Background: Cerebrospinal fluid (CSF) is in contact with brain parenchyma and ventricles, and its composition might influence the cellular physiology of oligodendrocyte progenitor cells (OPCs) thereby contributing to multiple sclerosis (MS) disease pathogenesis. Objective: To identify the transcriptional changes that distinguish the transcriptional response induced in proliferating rat OPCs upon exposure to CSF from primary progressive multiple sclerosis (PPMS) or relapsing remitting multiple sclerosis (RRMS) patients and other neurological controls. Methods: We performed gene microarray analysis of OPCs exposed to CSF from neurological controls, or definitive RRMS or PPMS disease course. R…

High anti-JCPyV serum titers coincide with high CSF cell counts in RRMS patients

2020

Background: Progressive multifocal leukoencephalopathy (PML) can in rare cases occur in natalizumab-treated patients with high serum anti-JCPyV antibodies, hypothetically due to excessive blockade of immune cell migration. Objective: Immune cell recruitment to the central nervous system (CNS) was assessed in relapsing-remitting multiple sclerosis (RRMS) patients stratified by low versus high anti-JCPyV antibody titers as indicator for PML risk. Methods: Cerebrospinal fluid (CSF) cell counts of 145 RRMS patients were quantified by flow cytometry. Generalized linear models were employed to assess influence of age, sex, disease duration, Expanded Disability Status Scale (EDSS), clinical/radiol…

The Relationship between Gray Matter Quantitative MRI and Disability in Secondary Progressive Multiple Sclerosis

2016

Purpose: In secondary progressive Multiple Sclerosis (SPMS), global neurodegeneration as a driver of disability gains importance in comparison to focal inflammatory processes. However, clinical MRI does not visualize changes of tissue composition outside MS lesions. This quantitative MRI (qMRI) study investigated cortical and deep gray matter (GM) proton density (PD) values and T1 relaxation times to explore their potential to assess neuronal damage and its relationship to clinical disability in SPMS. Materials and Methods: 11 SPMS patients underwent quantitative T1 and PD mapping. Parameter values across the cerebral cortex and deep GM structures were compared with 11 healthy controls, and…

A germline mutation in the platelet-derived growth factor receptor beta gene may be implicated in hereditary progressive mucinous histiocytosis.

2020

Are the new genetic tools for diagnosis of Wilson disease helpful in clinical practice?

2020

Summary The diagnosis of Wilson disease is not always easy. For many patients, a combination of tests reflecting disturbed copper metabolism may be needed. Testing for ATP7B variants has become part of the routine diagnostic approach. The methods of genetic testing include analysis of the 21 coding exons and intronic flanking sequences, in which exons with recurrent variants would be prioritised depending on the mutation frequency in the local population. If sequencing the entire ATP7B gene cannot identify 2 variants and the suspicion for Wilson disease is high, after reviewing the clinical data, WES (whole-exome sequencing) or WGS (whole-genome sequencing) could be applied. A workflow base…

Do individuals with higher cognitive ability play more strategically?

2016

Abstract This paper experimentally analyses the relationship between cognitive ability and strategic behaviour. In our experiment, individuals play in a sequential game, where computing the equilibrium is challenging. On completion of the game, we measure each player’s cognitive ability using Raven’s Progressive Matrices test. Our results reveal that the number of strategic decisions (played in the sequential game) increases significantly among those individuals with higher cognitive ability (measured by Raven’s test), compared to those with lower cognitive ability. These results clearly confirm that individuals with higher cognitive abilities play more strategically.

Failure by corrosion in PC bridges: A case history of a viaduct in Italy

2016

Purpose The paper illustrates a viaduct collapse due to corrosion phenomena. Moreover, a contribution to the issues related to both the control of existing structures and design methods to be followed for the construction of new buildings is provided. Design/methodology/approach The objectives were achieved by in situ observations and numerical analyses. The effects of corrosion phenomena are investigated, and the progressive collapse analysis is provided to be helpful in this case. Findings The damages induced by corrosion phenomena have caused the collapse of the viaduct taken in to account. The performed numerical analyses were able to reproduce the effects of corrosion in terms of redu…